NCCN T細胞淋巴瘤臨床實踐指南2017.2版（3）

目錄

原發性皮膚CD30陽性T細胞淋巴增殖性疾病（PCTLD）

PCTLD-1

概述&定義

註解：

a.Ralfkiaer E, Willemze R, Paulli M, Kadin ME. Primary cutaneous CD30-positive T-cell lymphoproliferative disorders. In: Swerdlow SH, Campo E, Harris NL, et al.,eds. WHO classification of tumours of haematopoietic and lymphoid tissues (ed 4th). Lyon: IARC; 2008:300-301.

b.Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375-2390.

c.Benner MF, Willemze R. Applicability and prognostic value of the new TNM classification system in 135 patients with primary cutaneous anaplastic large cell lymphoma. Arch Dermatol 2009;145:1399-1404.

d.Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood 2011;118:4024-4035.

e.由於重疊的免疫表型和形態，需要注意不要診斷淋巴結中的CD30 + T細胞作為HL。(Eberle FC, Song JY, Xi L, et al. Nodal involvement by cutaneous CD30-positive T-cell lymphoma mimicking classical Hodgkin lymphoma. Amer J Surg Pathol 2012;36:716-725.)

PCTLD-2

診斷

註解：

f.見「免疫分型和基因檢測在成熟B細胞和NK/T細胞淋巴瘤的鑒別診斷中的應用」（見《 B細胞淋巴瘤指南》）。

g.典型免疫表型：CD30+（>75%的細胞）、CD2/CD5/CD3的CD4+變型缺失、CD8+（

h.PC-ALCL和LyP中通常不存在ALK1陽性和t(2;5)易位。

i.TCR基因重排的結果應謹慎解釋。TCR克隆性重排也可見於非惡性疾患，也可能不會表現於所有的蕈樣肉芽腫/Sezary綜合征病例。對於某些病例，檢查皮膚、血液和/或淋巴結中的相同克隆可能會有幫助。

j.LyP不被認為是惡性疾病；但是，它與其他淋巴惡性腫瘤（蕈樣肉芽腫或PC-ALCL）存在關聯。只有存在相關全身性淋巴瘤懷疑時才進行LyP方面的分期研究。

PCTLD-3

檢查

註解：

e.由於免疫表型和形態的重疊，需要謹慎地避免將淋巴結中的CD30+ T細胞診斷為HL。(Eberle FC, Song JY, Xi L, et al.Nodal involvement by cutaneous CD30-positive T-cell lymphoma mimicking classical Hodgkin lymphoma.Amer J Surg Pathol 2012;36:716-725.)

j.LyP不被認為是惡性疾病；但是，它與其他淋巴惡性腫瘤（蕈樣肉芽腫或PC-ALCL）存在關聯。只有存在相關全身性淋巴瘤懷疑時才進行LyP方面的分期研究。

k.病灶大小和數量的監測將有助緩解評估。

l.考慮全身性ALCL、PC-ALCL區域巴結受累或轉化性MF淋巴結受累。

m.如果僅在引流淋巴結中，考慮PC-ALCL。

n.對於孕婦而言，許多作用於皮膚的治療或全身治療為禁用或其安全性不明。應參考具體用藥說明。

o.僅用於排除相關淋巴瘤。

PCTLD-4

原發皮膚及區域淋巴結受累皮膚ALCL的臨床路徑

註解：

p.最高達44%的病例可出現病灶進展。

q.見「放射治療原則」（LYMP-C）。

r.來自病例報告的有限數據（如貝沙羅汀）。

s.蕈樣肉芽腫可隨時間推移而出現；隨訪期間應持續進行全面的皮膚檢查。

t.從皮膚病治療中達到緩解和/或有臨床獲益患者應當考慮減量或維持治療以獲得最佳緩解持續時間。通常原方案對複發的患者仍然有效。部分緩解的患者在進入難治性疾病治療之前應當選擇主要治療列表中的其他方案以求提高療效。初始主要治療後疾病複發或持續的患者推薦參加臨床試驗。

PCTLD-5

LyP亞型的臨床路徑

註解：

r.來自病例報告的有限數據（如貝沙羅汀）。

u.Kempf W, Pfaltz K, Vermeer MH, et al.EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma.Blood 2011;118:4024-4035.

v.由於二次淋巴惡性腫瘤的風險很高，需要終身隨訪；隨訪期間應持續進行全面的皮膚檢查。

w.達到緩解和/或有臨床獲益的患者可考慮減量或維持治療以獲得最佳緩解持續時間。通常原方案對複發的患者仍然有效。部分緩解的患者在進入難治性疾病治療之前應當選擇主要治療列表中的其他方案以求提高療效。初始主要治療後疾病複發或持續的患者推薦參加臨床試驗。

PCTLD-A

參考文獻：

一般方法/治療概述

Kempf W, Pfaltz K, Vermeer MH et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30+ lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. Blood 2011;118:4024-4035.

Vergier B, Beylot-Barry M, Pulford K, et al. Statistical evaluation of diagnostic and prognostic features of CD30+ cutaneous lymphoproliferative disorders: a clinicopathologic study of 65 cases. Am J Surg Pathol 1998;22:1192-1202.

Liu HL, Hoppe RT, Kohler S, et al. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosos and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol 2003;49:1049-1058.

Woo DK, Jones CR, Vanoli-Stolz MN, et al. Prognostic factors in primary cutaneous anaplastic large cell lymphoma: characterization of clinical subset with worse outcome. Arch Dermatol 2009;145:667-674.

作用於皮膚的治療局部類固醇

Paul MA, Krowchuk DP, Hitchcock MG, et al. Lymphomatoid papulosis: successful weekly pulse superpotent topical corticosteroid therapy in three pediatric patients. Pediatr Dermatol 1996;13:501-506.

光療

Wantzin GL, Thomsen K. PUVA-treatment in lymphomatoid papulosis. Br J Dermatol 1982;107:687-690.

局部氮芥治療

Vonderheid EC, Tan ET, Kantor AF, et al. Long-term efcacy, curative potential, and carcinogenicity of topical mechloethamine chemotherapy in cutaneous T cell lymphoma. J Am Acad Dermatol 1989;20:416-428.

放療

Yu JB, McNi? JM, Lund MW et al. Treatment of primary cutaneous CD30+ anaplastic large cell lymphoma with radiation therapy. Int J Radiat Oncol Biol Phys 2008;70:1542-1545.

全身治療

甲氨蝶呤

Everett MA. Treatment of lymphomatoid papulosis with methotrexate. Br J Dermatol 1984;111:631.

Vonderheid EC, Sajjadian A, Kaden ME. Methotrexate is e?ective for lymphomatoid papulosis and other primary cutaneous CD30+ lymphoproliferative disorders. J Am Acad Dermatol 1996;34:470-481.

Fujita H, Nagatani T, Miyazawa M et al. Primary cutaneous anaplastic large cell lymphoma successfully treated with low-dose methotrexate. Eur J Dermatol 2008;18:360-361.

普拉曲沙

Horwitz SM, Kim YH, Foss F, et al. Identifcation of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T cell lymphoma. Blood 2012;119:4115-4122.

全身類視黃醇

Nakamura S, Hashimoto Y, Nishi K, et al. Primary cutaneous CD30+ lymphoproliferative disorder successfully treated with etretinate. Eur J Dermatol 2012;22:709-710. Krathen RA, Ward S, Duvic M. Bexarotene is a new treatment option for lymphomatoid papulosis. Dermatology 2003;206:142-147.

Wyss M, Dummer R, Dommann SN, et al. Lymphomatoid papulosis: treatment with recombinant interferon alfa-2a and etretinate. Dermatology 1995;190:288-291.

Sheehy JM, Catherwood M, Pettengeil R, et al. Sustained response of primary cutaneous CD30+ anaplastic large cell lymphoma to bexarotene and photopheresis. Leuk Lymphoma 2009;50:1389-1391.

干擾素

Proctor SJ, Jackson GH, Lennard AL, et al. Lymphotoid papulosis: response to treatment with recombinant interferon alfa-2b. J Clin Oncol 1992;10:170.

Yagi H, Tokura Y, Furukawa F, et al. Th2 cytokine mRNA expression in primary cutaneous CD30+ lymphoproliferative disorders: successful treatment with recombinant interferongamma. J Invest Dermatol 1996;107:827-832.

Schmuch M, Topar G, Illersperger B, et al. Therapeutic use of interferon-alpha for lymphomatoid papulosis. Cancer 2000;89:1603-1610.

Brentuximab vedotin

Duvic M, Tetzla? MT, Gangar P, et al. Results of a phase II trial of brentuximab vedotin for CD30+ cutaneous T-cell lymphoma and lymphomatoid papulosis. J Clin Oncol 2015;33:3759-65. Broccoli A, Derenzini E, Pellegrini C, et al. Complete response of relapsed systemic and cutaneous anaplastic large cell lymphoma using brentuximab vedotin: 2 case reports. Clin Lymphoma Myeloma Leuk 2013;13:493-495.

Mody K, Wallace JS, Stearns DM, et al. CD30+ cutaneous T cell lymphoma and response to brentuximab vedotin: 2 illustrative cases. Clin Lymphoma Myeloma Leuk 2014;13:319-323. Desai A, Telang GH, Olszewski AJ. Remission of primary cutaneous anaplastic large cell lymphoma after a brief course of brentuximab vedotin. Ann Hematol 2013;92:567-568.

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